ECHA rabbit pAb

Applications: WB
Reactivity: Human; Mouse;Rat
Source: Rabbit
Dilution: WB 1:500-2000
Immunogen: Synthesized peptide derived from human ECHA AA range: 276-326
Storage_stability: -20°C/1 year
Clonality: Polyclonal
Isotype: IgG
Concentration: 1 mg/ml
Observed_band(KD): 85kD
Human_gene_id: 3030
Human_swiss_prot_no: P40939
Subcellular_location: Mitochondrion . Mitochondrion inner membrane . Protein stability and association with mitochondrion inner membrane do not require HADHB. .
Other_name: Trifunctional enzyme subunit alpha, mitochondrial (78 kDa gastrin-binding protein) (TP-alpha) [Includes: Long-chain enoyl-CoA hydratase (EC 4.2.1.17); Long chain 3-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.211)]
Background: This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008],